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HTLV-1-associated myelopathy-tropical spastic paraparesis(HAM)

MedGen UID:: 18298
•Concept ID:: C0030481
•: Disease or Syndrome

Synonyms:	FAMILIAL SPASTIC PARAPARESIS, HTLV-1-ASSOCIATED; HAM; Human T-cell leukemia virus type 1 associated myelopathy/tropical spastic paraparesis; MYELOPATHY, HTLV-1-ASSOCIATED; Paraparesis, Tropical Spastic; Tropical spastic paraparesis (formerly)
SNOMED CT:	Tropical spastic paraplegia (714279000); HTLV-I-associated myelopathy (714279000); Tropical spastic paraparesis (714279000); Human T-cell lymphotropic virus 1-associated myelopathy (714279000); Myelopathy caused by Human T-lymphotropic virus 1 (714279000)
Modes of inheritance:	Not genetically inherited MedGen UID: 988794 •Concept ID: CN307044 • Finding Source: Orphanet clinical entity without genetic inheritance. See: This record Not genetically inherited (Orphanet)

Monarch Initiative:	MONDO:0008039
OMIM®:	159580
Orphanet:	ORPHA289326

Definition

A progressive chronic inflammatory disease of the central nervous system with the aetiologic agent Human T cell lymphotropic virus type I (HTLV-I), the disease is characterised by unremitting myelopathic symptoms such as spastic paraparesis, bowel and/or bladder dysfunction and sensory changes of the lower limbs. [from SNOMEDCT_US]

Clinical features

From HPO

Spastic paraparesis

MedGen UID:: 52432
•Concept ID:: C0037771
•: Sign or Symptom

Mild or moderate loss of motor function accompanied by spasticity in the lower extremities. This condition is a manifestation of CENTRAL NERVOUS SYSTEM DISEASES that cause injury to the motor cortex or descending motor pathways.

See: Feature record | Search on this feature

Myelopathy

MedGen UID:: 11550
•Concept ID:: C0037928
•: Disease or Syndrome

A non neoplastic or neoplastic disorder that affects the spinal cord.

See: Feature record | Search on this feature

Abnormal pyramidal sign

MedGen UID:: 68582
•Concept ID:: C0234132
•: Sign or Symptom

Functional neurological abnormalities related to dysfunction of the pyramidal tract.

See: Feature record | Search on this feature

Abnormality of the nervous system

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVHTLV-1-associated myelopathy-tropical spastic paraparesis

Infection
- viral disease
  - RNA Virus Infections
    - Disease due to Retroviridae
      - Deltaretrovirus infections
        Human T-lymphotropic virus 1 infectious disease
        HTLV-1-associated myelopathy-tropical spastic paraparesis

Follow this link to review classifications for HTLV-1-associated myelopathy-tropical spastic paraparesis in Orphanet.

Professional guidelines

PubMed

Advances in the treatment of human T-cell lymphotropic virus type-I associated myelopathy.

Mashkani B, Jalili Nik M, Rezaee SA, Boostani R
Expert Rev Neurother 2023 Jul-Dec;23(12):1233-1248. Epub 2023 Dec 15 doi: 10.1080/14737175.2023.2272639. PMID: 37933802

Mogamulizumab for Treatment of Human T-lymphotropic Virus Type 1-Associated Myelopathy/Tropical Spastic Paraparesis: A Single-Center US-based Series.

Meyerowitz EA, Mukerji SS, Kyle Harrold G, Erdil RM, Chen ST, Rudmann EA, Tsibris A, Venna N, Robbins GK
Clin Infect Dis 2023 Sep 18;77(6):851-856. doi: 10.1093/cid/ciad281. PMID: 37157862 Free PMC Article

Highly active antiretroviral treatment against STLV-1 infection combining reverse transcriptase and HDAC inhibitors.

Afonso PV, Mekaouche M, Mortreux F, Toulza F, Moriceau A, Wattel E, Gessain A, Bangham CR, Dubreuil G, Plumelle Y, Hermine O, Estaquier J, Mahieux R
Blood 2010 Nov 11;116(19):3802-8. Epub 2010 Jun 29 doi: 10.1182/blood-2010-02-270751. PMID: 20587783

See all (12)

Recent clinical studies

Etiology

Cognitive deficits in HTLV-1 patients.

Kamrani M, Saryazdi S, Zemorshidi F, Khadem-Rezaiyan M, Behravan G
J Neurovirol 2023 Aug;29(4):416-424. Epub 2023 May 19 doi: 10.1007/s13365-023-01139-x. PMID: 37204651

Cardiovascular risk factors and brain white matter lesions in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

Filho ABV, Puccioni-Sohler M
J Neurovirol 2022 Feb;28(1):168-171. Epub 2022 Mar 2 doi: 10.1007/s13365-022-01063-6. PMID: 35235153

Spasticity distribution and severity in individuals with HTLV-1-associated myelopathy/tropical spastic paraparesis.

Sales M, de Almeida Scaldaferri GB, Barbosa Dos Santos JI, Melo A, da Silva Ribeiro NM
J Neurovirol 2021 Dec;27(6):857-863. Epub 2020 Oct 6 doi: 10.1007/s13365-020-00911-7. PMID: 33021698

Impact of fractures and orthopedic surgeries in patients with HTLV-1 associated myelopathy/tropical spastic paraparesis.

Julca-Copello P, Schwalb A, Cachay R, Tipismana M, Alvarez C, Mejía F, González-Lagos E, Gotuzzo E
Rev Soc Bras Med Trop 2020;53:e20200101. Epub 2020 Sep 11 doi: 10.1590/0037-8682-0101-2020. PMID: 32935782 Free PMC Article

HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis Is Not Associated with SNP rs12979860 of the IL-28B Gene.

Vallinoto AC, Santana BB, Sá KS, Ferreira TC, Sousa RC, Azevedo VN, Feitosa RN, Machado LF, Ishak MO, Ishak R
Mediators Inflamm 2015;2015:804167. Epub 2015 Nov 1 doi: 10.1155/2015/804167. PMID: 26609200 Free PMC Article

See all (199)

Diagnosis

Cognitive deficits in HTLV-1 patients.

Kamrani M, Saryazdi S, Zemorshidi F, Khadem-Rezaiyan M, Behravan G
J Neurovirol 2023 Aug;29(4):416-424. Epub 2023 May 19 doi: 10.1007/s13365-023-01139-x. PMID: 37204651

Spasticity distribution and severity in individuals with HTLV-1-associated myelopathy/tropical spastic paraparesis.

Sales M, de Almeida Scaldaferri GB, Barbosa Dos Santos JI, Melo A, da Silva Ribeiro NM
J Neurovirol 2021 Dec;27(6):857-863. Epub 2020 Oct 6 doi: 10.1007/s13365-020-00911-7. PMID: 33021698

An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.

Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825

Impact of fractures and orthopedic surgeries in patients with HTLV-1 associated myelopathy/tropical spastic paraparesis.

HTLV-1 Associated Neurological Disorders.

Khan MY, Khan IN, Farman M, Al Karim S, Qadri I, Kamal MA, Al Ghamdi K, Harakeh S
Curr Top Med Chem 2017;17(12):1320-1330. doi: 10.2174/1568026616666161222141318. PMID: 28017149

See all (130)

Therapy

An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.

Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825

Human T-lymphotropic virus type 1 (HTLV-1) and cellular immune response in HTLV-1-associated myelopathy/tropical spastic paraparesis.

Nozuma S, Kubota R, Jacobson S
J Neurovirol 2020 Oct;26(5):652-663. Epub 2020 Jul 23 doi: 10.1007/s13365-020-00881-w. PMID: 32705480 Free PMC Article

Immunovirological markers in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).

Enose-Akahata Y, Jacobson S
Retrovirology 2019 Nov 29;16(1):35. doi: 10.1186/s12977-019-0499-5. PMID: 31783764 Free PMC Article

HTLV-1 Associated Neurological Disorders.

Khan MY, Khan IN, Farman M, Al Karim S, Qadri I, Kamal MA, Al Ghamdi K, Harakeh S
Curr Top Med Chem 2017;17(12):1320-1330. doi: 10.2174/1568026616666161222141318. PMID: 28017149

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP): still an obscure disease.

Pillat MM, Bauer ME, de Oliveira AC, Ulrich H, Casseb J
Cent Nerv Syst Agents Med Chem 2011 Dec 1;11(4):239-45. doi: 10.2174/1871524911106040239. PMID: 22300227

See all (62)

Prognosis

T cell receptor repertoire analysis in HTLV-1-associated diseases.

Clauze A, Enose-Akahata Y, Jacobson S
Front Immunol 2022;13:984274. Epub 2022 Sep 15 doi: 10.3389/fimmu.2022.984274. PMID: 36189294 Free PMC Article

An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.

Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825

Mortality and risk of progression to adult T cell leukemia/lymphoma in HTLV-1-associated myelopathy/tropical spastic paraparesis.

Nagasaka M, Yamagishi M, Yagishita N, Araya N, Kobayashi S, Makiyama J, Kubokawa M, Yamauchi J, Hasegawa D, Coler-Reilly ALG, Tsutsumi S, Uemura Y, Arai A, Takata A, Inoue E, Hasegawa Y, Watanabe T, Suzuki Y, Uchimaru K, Sato T, Yamano Y
Proc Natl Acad Sci U S A 2020 May 26;117(21):11685-11691. Epub 2020 May 11 doi: 10.1073/pnas.1920346117. PMID: 32393644 Free PMC Article

Role of HTLV-1 orf-I encoded proteins in viral transmission and persistence.

Sarkis S, Galli V, Moles R, Yurick D, Khoury G, Purcell DFJ, Franchini G, Pise-Masison CA
Retrovirology 2019 Dec 18;16(1):43. doi: 10.1186/s12977-019-0502-1. PMID: 31852543 Free PMC Article

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) inflammatory network.

Goncalves DU, Proietti FA, Barbosa-Stancioli EF, Martins ML, Ribas JG, Martins-Filho OA, Teixeira-Carvalho A, Peruhype-Magalhães V, Carneiro-Proietti AB
Inflamm Allergy Drug Targets 2008 Jun;7(2):98-107. doi: 10.2174/187152808785107642. PMID: 18691139

See all (72)

Clinical prediction guides

Cognitive deficits in HTLV-1 patients.

Kamrani M, Saryazdi S, Zemorshidi F, Khadem-Rezaiyan M, Behravan G
J Neurovirol 2023 Aug;29(4):416-424. Epub 2023 May 19 doi: 10.1007/s13365-023-01139-x. PMID: 37204651

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) versus adult T-cell leukemia/lymphoma (ATLL).

Zarei-Ghobadi M, Sheikhi M, Teymoori-Rad M, Yaslianifard S, Norouzi M, Yaslianifard S, Faraji R, Farahmand M, Bayat S, Jafari M, Mozhgani SH
BMC Res Notes 2021 Mar 23;14(1):109. doi: 10.1186/s13104-021-05521-y. PMID: 33757561 Free PMC Article

An update on human T-cell leukemia virus type I (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) focusing on clinical and laboratory biomarkers.

Yamauchi J, Araya N, Yagishita N, Sato T, Yamano Y
Pharmacol Ther 2021 Feb;218:107669. Epub 2020 Aug 21 doi: 10.1016/j.pharmthera.2020.107669. PMID: 32835825

Mogamulizumab (Anti-CCR4) in HTLV-1-Associated Myelopathy.

Sato T, Coler-Reilly ALG, Yagishita N, Araya N, Inoue E, Furuta R, Watanabe T, Uchimaru K, Matsuoka M, Matsumoto N, Hasegawa Y, Yamano Y
N Engl J Med 2018 Feb 8;378(6):529-538. doi: 10.1056/NEJMoa1704827. PMID: 29414279

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP): still an obscure disease.

Pillat MM, Bauer ME, de Oliveira AC, Ulrich H, Casseb J
Cent Nerv Syst Agents Med Chem 2011 Dec 1;11(4):239-45. doi: 10.2174/1871524911106040239. PMID: 22300227

See all (184)

Recent systematic reviews

Cerebral and spinal cord changes observed through magnetic resonance imaging in patients with HTLV-1-associated myelopathy/tropical spastic paraparesis: a systematic review.

Bastos Ferreira AP, do Nascimento ADFS, Sampaio Rocha-Filho PA
J Neurovirol 2022 Feb;28(1):1-16. Epub 2022 Jan 3 doi: 10.1007/s13365-021-01043-2. PMID: 34981435

Intrinsic and Extrinsic Cell Apoptotic Pathways in Patients with HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis: A Systematic Review.

Bastos Ferreira AP, Cassilhas APP, Moura P, Sampaio Rocha-Filho PA
Viral Immunol 2021 Jul-Aug;34(6):380-391. Epub 2021 Jan 19 doi: 10.1089/vim.2020.0131. PMID: 33470891

Population-based prevalence of human T-lymphotropic virus type 1 in sub-Saharan Africa: a systematic review and meta-analysis.

Ngoma AM, Omokoko MD, Mutombo PB, Mvika ES, Muwonga JM, Nollet KE, Ohto H
Trop Med Int Health 2019 Nov;24(11):1277-1290. Epub 2019 Sep 30 doi: 10.1111/tmi.13305. PMID: 31465629

Current approaches for detection of human T-lymphotropic virus Type 1: A systematic review.

Fani M, Rezayi M, Meshkat Z, Rezaee SA, Makvandi M, Abouzari-Lotf E, Ferns GA
J Cell Physiol 2019 Aug;234(8):12433-12441. Epub 2019 Jan 11 doi: 10.1002/jcp.28087. PMID: 30633358

Early Onset of HTLV-1 Associated Myelopathy/Tropical Spastic Paraparesis (HAM/TSP) and Adult T-cell Leukemia/Lymphoma (ATL): Systematic Search and Review.

Oliveira PD, Kachimarek AC, Bittencourt AL
J Trop Pediatr 2018 Apr 1;64(2):151-161. doi: 10.1093/tropej/fmx039. PMID: 28582585

See all (5)

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HTLV-1-associated myelopathy-tropical spastic paraparesis(HAM)

Definition

Clinical features

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Molecular resources

Consumer resources

Reviews

Related information

Recent activity